Early diagnosis and appropriate treatment translates to more lives, eyes, and vision being saved

Dr Swathi Kaliki, Head – The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad highlights the dangers of retinoblastoma, the need to diagnose it on time to improve survival rates, the various measures undertaken by LV Prasad Eye Institute to spread awareness about the disease and offer quality treatment etc, in an interaction with Lakshmipriya Nair

How common is the incidence of retinoblastoma in India?

The incidence of retinoblastoma is one in 15,000 to 18,000 live births. As per studies from worldwide, there is no difference in the incidence of retinoblastoma based on the region of origin. However, in India we see more number of cases probably due to higher population rate.

What are the causes for its occurrence? How curable is the disease?

Retinoblastoma occurs due to mutation in chromosome 13q14. This can be a sporadic mutation (90 to 95 per cent) or inherited from one of the parent (5 to 10 per cent).

Retinoblastoma treatment is a great success story. The survival rate of children with retinoblastoma was only five per cent about 50 years ago, but today with improved treatment modalities, the survival rate is 95 per cent. The death is mostly related to delayed diagnosis and treatment.

Any measures which can help in its prevention?

We can certainly not prevent the genetic mutation but should create awareness about early detection and appropriate treatment. Patients with retinoblastoma have a risk of passing on the genetic mutation to their offsprings. They should undergo genetic testing and be given adequate genetic counselling so that the risk can be assessed in the pre-natal period and the delivery can be planned appropriately. And those who already have delivered a child, the child should be screened for retinoblastoma by a trained ocular oncologist immediately after delivery.

What are the challenges in diagnosing the disease? Are Indian children more susceptible to it due to certain reasons? If yes, kindly elaborate.

The challenge in India is early detection of retinoblastoma. Most children are referred to us when the disease is already advanced, and thus minimising the chances of saving the eye and vision, although the life can be saved in most cases. All registered medical practitioners and nurses in every urban or rural area should be made aware of the early signs of this disease so that they can be referred to us in the early stage, so as to achieve the goals of saving life, eye, and vision in these children. The parents should be made aware of this deadly cancer so that they seek medical help at the earliest.

What is the line of treatment generally adopted to cure retinoblastoma?

Retinoblastoma can be intraocular (within the eye) or have extra ocular extension (tumour extending outside the eye). If intraocular, then it can be grouped into five groups. Group A and B can be treated with transpupillary thermotherapy or cryotherapy. Group C and D can be treated with systemic chemotherapy or intra-arterial chemotherapy. Group E might need enucleation (removal of the eye). If there is extra ocular extension, the child needs combination treatment of systemic chemotherapy, enucleation, and radiotherapy.

What are the new technologies that have aided in diagnosis and treatment of this disease?

Prenatal diagnosis of retinoblastoma (diagnosis of retinoblastoma before birth) is possible with amniocentesis or chorionic villus sampling and genetic testing. In later stages of pregnancy, the diagnosis can be done by ultrasonography. After birth, a good clinical examination helps in accurate diagnosis. Ultrasonography, CT scan, MRI scan, optical coherence tomography also aid in diagnosis.

The treatment modalities of retinoblastoma include transpupillary thermotherapy, cryotherapy, plaque radiotherapy, external beam radiotherapy, systemic chemotherapy, intra-arterial chemotherapy, periocular chemotherapy, and intravitreal chemotherapy. Intra-arterial chemotherapy is the new form of treatment where chemotherapy is directly delivered to the affected eye by arterial route.

What are the measures needed to spread awareness about this ailment and its impact?

Electronic and print media play a big role in spreading awareness about retinoblastoma. Regular news bytes and patient stories are needed to make general public aware of the disease. We at LV Prasad Eye Institute hold regular awareness programmes to train our vision technicians, optometrists, anganwadi workers, and all health care staff in recognising the disease early. Our vision technicians who work in the remote areas are able to identify the disease and refer patients to The Operation Eyesight Universal Institute for Eye Cancer at LV Prasad Eye Institute, so that early treatment can be offered.

Early diagnosis and appropriate treatment translates to more lives, eyes, and vision being saved.

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